Self‐inflicted injury in a case of Hallervorden‐Spatz disease

Sheehy, Evelyn; Longhurst, P; Pool, D M; Dandekar, Mitali

doi:10.1111/j.1365-263x.1999.00149.x

Cited by 15 publications

(10 citation statements)

References 17 publications

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“…SIB was generally connected to diseases with neuropsychiatric symptoms and infectious diseases or the patient was very young in the literature [ 1 – 3 , 5 , 6 ]. In this case, a systemically healthy and severely burned child who was undermedicated was self-mutilated by extracting his teeth.…”

Section: Discussionmentioning

confidence: 99%

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Autoextraction of Permanent Incisors and Self-Inflicted Orodental Trauma in a Severely Burned Child

Keleş

Doğusal

Sönmez

2015

Case Reports in Dentistry

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Autoextraction is one type of self-injurious behaviour. In the literature, self-injurious behaviours are observed in syndromes and genetic conditions. However, to the best of our knowledge, SIB and autoextraction in a severely burned patient have not been reported to date. This report describes the self-inflicted trauma and autoextraction in a severely burned child, and the management of the child during and after burn treatment.

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Section: Discussionmentioning

confidence: 99%

“…In the literature, there are various types of self-inflicted behaviours. SIB has been observed in a child affected with Hallervorden-Spatz disease, a rare neurodegenerative condition that is characterized by progressive dystonia, rigidity, and mental retardation [ 6 ].…”

Section: Introductionmentioning

confidence: 99%

Autoextraction of Permanent Incisors and Self-Inflicted Orodental Trauma in a Severely Burned Child

Keleş

Doğusal

Sönmez

2015

Case Reports in Dentistry

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“…Based on limited case reports, most dsytonia get relieved after intravenous anesthetics and the airway can be maintained. Propofol, thiopental, fentanyl, remifentanil, N 2 O, volatile anesthetics and several neuromuscular blocking agents had been used without adverse effect [4,5,[12][13][14].…”

Section: Discussionmentioning

confidence: 99%

Intrathecal Baclofen Facilitated Postanesthetic Tracheal Extubation in a Dystonic Patient Associated with Neurodegeneration of Brain Iron Accumulation (Hallervorden-Spatz Disease)

Lee¹,

Chu²,

Chuang³

et al. 2011

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Patients with Hallervorden-Spatz disease may be confronted by invasive procedure, like gastrostomy and thalamotomy for care of the status of extreme dystonia and rigidity. This rare disorder possesses potential perioperative risks, such as difficult airway management, aspiration pneumonia, hyperpyrexia, dehydration, acute renal failure, and postoperative pulmonary insufficiency. As patients were usually in the state of uncontrollable dystonia and rigidity, delayed endotracheal extubation had been suggested by several previous case reports. We report the anesthetic management of a 34-year-old Hallervorden-Spatz disease woman scheduled for intrathecal baclofen pump implantation under general anesthesia. Preoperatively she had suffered from severe dystonia, confusion and repeated attacks of respiratory insufficiency and pneumonia requiring mechanical ventilation and critical care. Anesthesia was induced uneventfully and the catheter of the pump reached the CSF via T2 interspace. Intraoperative intrathecal baclofen injection mildly relieved her severe dystonia and rigidity. Hemodynamics and arterial blood gas were improved than preoperative. She was extubated after consciousness was regained. Postanesthetic visit demonstrated a fair outcome. Whether this treatment applies to every case confronted by general anesthesia needs to be elucidated. Nevertheless, we suggest intrathecal baclofen may help postanesthetic pulmonary care by attenuating dystonia and rigidity and can be an adjuvant for anesthesia care in patients with Hallervorden-Spatz disease

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“…[ 3 ] Only one case is reported with self-mutilation in the world literature to our knowledge. [ 4 ] Based on the age of onset they are grouped into: Late infantile – till the age of 6 years; postinfantile – 7 to 15 years; adult – 22 to 64 years. Course is variable from rapid death to a prolonged stable course.…”

Section: Introductionmentioning

confidence: 99%

“…Here we report a case of neurodegeneration with brain iron accumulation of late infantile type with severe self-mutilation of lips and episodic ophisthotonic posturing of the trunk with extension to both lower limbs precipitated by any attempted action. [ 1 4 5 6 ]…”

Section: Introductionmentioning

confidence: 99%

Self-mutilation in neurodegeneration with brain iron accumulation

2015

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Neurodegeneration with brain iron accumulation (NBIA) is the term applied to a heterogeneous group of disorders resulting in iron deposition in the basal ganglia. Well-known phenotypic features are progressive regression with extra pyramidal involvement and a variable course. A 10-year-old child born to consanguineous parents presented with progressive generalized opisthotonic dystonia, retrocollis, oromandibular dyskinesias, apraxia for swallowing, optic atrophy and severe self-mutilation of lips. MR imaging showed brain iron accumulation. Other causes of self-mutilation were excluded. Early infantile onset, ophisthotonic dystonia with oromandibular dyskinesias and characteristic MR images are suggestive of NBIA. There is only one case reported in the literature of self-mutilation in this condition.

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Self‐inflicted injury in a case of Hallervorden‐Spatz disease

Cited by 15 publications

References 17 publications

Autoextraction of Permanent Incisors and Self-Inflicted Orodental Trauma in a Severely Burned Child

Autoextraction of Permanent Incisors and Self-Inflicted Orodental Trauma in a Severely Burned Child

Intrathecal Baclofen Facilitated Postanesthetic Tracheal Extubation in a Dystonic Patient Associated with Neurodegeneration of Brain Iron Accumulation (Hallervorden-Spatz Disease)

Self-mutilation in neurodegeneration with brain iron accumulation

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